Brief Report: Challenging Behaviors in Toddlers and Preschoolers with Angelman, Prader-Willi, and Williams Syndromes
- PMID: 30542941
- PMCID: PMC7156277
- DOI: 10.1007/s10803-018-3853-x
Brief Report: Challenging Behaviors in Toddlers and Preschoolers with Angelman, Prader-Willi, and Williams Syndromes
Abstract
Children with neurogenetic syndromes (NGS) experience comorbid challenging behaviors and psychopathology. We examined challenging behaviors in 86 toddlers and preschoolers across three NGS [Angelman syndrome (AS), Prader-Willi syndrome (PWS), and Williams syndrome (WS)] and 43 low-risk controls (LRC), using the Child Behavior Checklist for Ages 1½-5. Challenging behavior profiles differed across NGS, with generally elevated behaviors in AS and WS, but not PWS, relative to LRC. Withdrawn and autism spectrum symptoms were particularly elevated in AS. Although several profiles were similar to those previously reported in older children and adults, we also observed inconsistencies that suggest non-linear developmental patterns of challenging behaviors. These findings underscore the importance of characterizing early challenging behaviors to inform atypical phenotypic development and targeted intervention.
Keywords: Angelman syndrome; Challenging behavior; Child Behavior Checklist; Early childhood; Prader–Willi syndrome; Williams syndrome.
Conflict of interest statement
Conflict of interest: The authors declare that they have no conflict of interest.
Figures
Similar articles
-
Using generalizability theory to evaluate the comparative reliability of developmental measures in neurogenetic syndrome and low-risk populations.J Neurodev Disord. 2020 Jun 5;12(1):16. doi: 10.1186/s11689-020-09318-1. J Neurodev Disord. 2020. PMID: 32503425 Free PMC article.
-
Specificity of Early Childhood Hyperphagia Profiles in Neurogenetic Conditions.Am J Intellect Dev Disabil. 2024 May 1;129(3):175-190. doi: 10.1352/1944-7558-129.3.175. Am J Intellect Dev Disabil. 2024. PMID: 38657964
-
Maternal parenting stress in families with a child with Angelman syndrome or Prader-Willi syndrome.J Intellect Dev Disabil. 2010 Sep;35(3):165-74. doi: 10.3109/13668250.2010.499101. J Intellect Dev Disabil. 2010. PMID: 20809878
-
Behavioral phenotypes in genetic syndromes: genetic clues to human behavior.Adv Pediatr. 2002;49:59-86. Adv Pediatr. 2002. PMID: 12214780 Review.
-
[Neuropsychiatric aspects of Prader-Willi syndrome – a review].Z Kinder Jugendpsychiatr Psychother. 2018 May;46(3):238-246. doi: 10.1024/1422-4917/a000530. Epub 2017 Jun 14. Z Kinder Jugendpsychiatr Psychother. 2018. PMID: 28613110 Review. German.
Cited by
-
Anxiety in Young Children with Williams Syndrome: A Longitudinal Study.Children (Basel). 2025 Aug 21;12(8):1098. doi: 10.3390/children12081098. Children (Basel). 2025. PMID: 40868549 Free PMC article.
-
When they just don't sleep: differential impacts of reduced child sleep on depression, anxiety, and stress among caregivers of children with and without neurogenetic syndromes.Front Psychiatry. 2024 Apr 19;15:1352881. doi: 10.3389/fpsyt.2024.1352881. eCollection 2024. Front Psychiatry. 2024. PMID: 38707621 Free PMC article.
-
Internalising and Externalising Symptoms and Their Association with the Family Environment in Young Children with Williams Syndrome: A Longitudinal Study.Children (Basel). 2023 Oct 23;10(10):1717. doi: 10.3390/children10101717. Children (Basel). 2023. PMID: 37892382 Free PMC article.
-
Perception of psychosocial burden in mothers of children with rare pediatric neurological diseases.Sci Rep. 2025 Feb 21;15(1):6295. doi: 10.1038/s41598-025-87251-w. Sci Rep. 2025. PMID: 39984547 Free PMC article.
References
-
- Achenbach TM, & Rescorla LA (2000). Manual for the ASEBA preschool forms and profiles. Burlington, VT: University of Vermont Research Center for Children, Youth, and Families.
-
- Åkefeldt A, & Gillberg C (1999). Behavior and personality characteristics of children and young adults with Prader-Willi syndrome: A controlled study. Journal of the American Academy of Child and Adolescent Psychiatry, 38(6), 761–769. - PubMed
-
- Alexander RC (2016). Prader-Willi syndrome In Rubin IL, Merrick J, Greydanus DE, & Patel DR (Eds.), Health care for people with intellectual and developmental disabilities across the lifespan (pp. 779–798). Switzerland: Springer International Publishing.
-
- Annaz D, Hill CM, Ashworth A, Holley S, & Karmiloff-Smith A (2011). Characterisation of sleep problems in children with Williams syndrome. Research in Developmental Disabilities, 32(1), 164–169. - PubMed
MeSH terms
Grants and funding
LinkOut - more resources
Full Text Sources
Medical
Research Materials